Berdel P, Gravius S, Goldmann G, Pennekamp P, Oldenburg J, Seuser A, Wirtz D C
Klinik und Poliklinik für Orthopädie und Unfallchirurgie, Rheinische Friedrich-Wilhelms-Universität Bonn, Sigmund-Freud-Str. 25, 53127 Bonn, Germany.
Hamostaseologie. 2008 Oct;28 Suppl 1:S45-9.
Acute compartment syndrome is a complication in which microcirculation is impaired due to increased tissue pressure within a confined (osteo-fibrous) space and leads to neuromuscular dysfunction. A serious complication of haemophilia is the development of inhibitors. In this case the immune system produces antibodies to factor VIII or IX during substitution therapy of haemophilia A or B. These antibodies are directed against both, the substituted and the endogenous factors.
A man (age: 81 years) with originally moderate haemophilia A who at the age of 63 developed an inhibitor during treatment of a bleeding event. Painful swelling in the left forearm occurred without any recollection of trauma, and failed to subside under factor substitution initially performed by the patient. This finding necessitated emergency fasciotomy of the forearm flexor compartment.
In order to keep the complication rate as low as possible in the presence of hemophilia with inhibitors, the patients should only be treated in a specially equipped interdisciplinary treatment center.
